Cardiac involvement in Lyme disease: manifestations and management

Cardiac involvement in Lyme disease may manifest as atrioventricular block, myopericarditis, and left ventricular dysfunction. Diagnosis depends on recognition of the systemic nature of Lyme disease, including cardiac involvement, and its natural history. Serologic tests that are both sensitive and specific may aid in diagnosis. Although current recommendations for the treatment of Lyme disease with carditis include antibiotics and salicylates or corticosteroids, these types of therapy have not been unequivocally demonstrated to alter the natural history of cardiac involvement. Supportive therapy may necessitate temporary transvenous cardiac pacing in symptomatic patients.     

Rhizomelic chondrodysplasia punctata: 16-year follow-up of a child from birth

A boy with rhizomelic chondrodysplasia punctata, diagnosed on the laboratory evidence of a high plasma concentration of phytanic acid and a low erythrocyte concentration of plasmalogens, has been followed from birth to the age of 16 years. The clinical pattern (somatic, skeletal and neurological) tallies with published findings in this disease. Unusual findings are the associated epilepsy, confirmed by EEG, and the long survival. CT brain scan and MRI showed cortical and subcortical atrophy but not gyral abnormalities or demyelination.

---

Sommario Viene descritto il caso di un bambino affetto da condrodisplasia punctata rizomelica seguito dalla nascita fino ai 16 anni. La diagnosi clinica, efettuata in base alle caratteristiche somatiche, scheletriche e neurologiche del paziente è stata confermata dai reperti di laboratorio (alta concentrazione plasmatica di acido fitanico, bassa concentrazione di plasmalogeni eritrocitari). Come correlati inusuali del quadro clinico vanno segnalati l'associazione con epilessia e a la lunga sopravvivenza. La TAC e la RNM cerebrale hannovaltresì documentato atrofia corticale e sottocorticale ma non anomalie della girazione o demielinizzazione.

     

Lymphoscintigraphy in lymphedema: an aid to microsurgery

The role of lymphoscintigraphy, performed with 99mTc-labeled antimony sulfur colloid, in the diagnosis of lymphedema and as a test for selection of patients for microvascular operation was evaluated in 32 patients with primary and secondary lymphedema and four patients with other causes of leg edema. Lymphoscintigraphy clearly demonstrated if edema was of lymphatic origin. Five different image patterns were identified; abnormal image patterns could not be predicted from clinical history or physical findings. Quantitative evaluation of removal of the radioactive colloid from the injection site and appearance in lymph node sites and liver was of limited usefulness. Nine patients underwent various surgical procedures before or after lymphoscintigraphy. Lympho-venous anastomoses were possible only in patients who had patent lymph channels visible on lymphoscintigrams. Based on initial experience, lymphoscintigraphy seems to be useful to select patients for microvascular operation.     

Allografts of CNS tissue possess a blood-brain barrier. II. Angiogenesis in solid tissue and cell suspension grafts

Angiogenesis and patency of blood vessels were analyzed qualitatively in solid CNS and peripheral tissue syngeneic, allogeneic, and xenogeneic grafts and in individual cell suspension grafts of astrocytes, fibroblasts, PC12, and three additional tumor cell lines placed intracerebrally in adult host mice. Postgrafting survival times were 1 day through 4 weeks. The patency of graft vessels was determined in sections from immersion-fixed tissues incubated to reveal the endogenous peroxidase activity of host red cells trapped within the lumen of blood vessels. Additionally, horseradish peroxidase (HRP) was administered intravenously to live hosts; HRP labels host brain and graft vessels on the luminal surface and reveals the presence or absence of a blood-brain barrier (BBB) within the grafts. The origins of blood vessels supplying solid tissue xenografts were identified immunohistochemically with primary antibodies against host (athymic AKR mice) and donor (fetal Lewis rats) major histocompatibility complex (MHC) class I. Blood vessels supplying solid CNS grafts at 1-7 days post-transplantation were identified ultrastructurally and possessed interendothelial tight junctional complexes; however, they were not perfused with either host blood or blood-borne HRP prior to 8 days. Graft vessels at 10 days were outlined consistently by peroxidase-positive red cells in immersion-fixed material and labeled with blood-borne HRP. These vessels provided a BBB to the circulating HRP and exhibited interendothelial tight junctions. Evidence of angiogenesis within solid anterior pituitary grafts and the variety of cell suspension grafts was obtained prior to 3 days post-transplantation in immersion-fixed preparations; the vessels, with the notable exception of those supplying astrocyte cell suspensions, failed to present a BBB to blood-borne peroxidase. Endothelia in the solid pituitary allografts and the PC12 cell grafts were highly fenestrated and exhibited open interendothelial junctions; those in the tumor and fibroblast cell grafts, for the most part, appeared nonfenestrated, and many possessed open interendothelial junctional complexes. Immunostaining for host and donor MHC class I revealed that donor blood vessels predominate over host vessels in CNS xenografts and supply pituitary xenografts exclusively; in both preparations, donor vessels were not identified within the host CNS. Because cell suspension grafts were derived from endothelia-free preparations grown in culture, blood vessels supplying these grafts were necessarily of host CNS origin and manifested a morphological transformation from a BBB to a non-BBB endothelium. The data suggest that angiogenesis in solid CNS grafts placed into the adult host CNS, compared to similarly placed solid peripheral tissue/cell suspension grafts, is not rapid.(ABSTRACT TRUNCATED AT 400 WORDS)     

Effect of bezafibrate on low levels of high density lipoprotein cholesterol in normolipidemia. Study in coronary patients and their first degree relatives

A pharmacological study was carried out in a group of 20 subjects composed of 13 coronary patients and 7 normal close relatives (first degree) who were considered at a high risk for coronary heart disease (CHD) because of their low levels of high density lipoprotein cholesterol (HDLch) (mean +/- SD: 34.1 +/- 5.2 mg/dl) and their high total cholesterol/HDLch (Tch/HDLch) ratio (mean +/- SD:6.7 +/- 1.1), despite their normal serum lipid values. With the purpose of normalizing these parameters they were submitted to a 4-month treatment with bezafibrate, a hypolipidemic agent which has a known effect in increasing HDLch and in decreasing the Tch/HDLch ratio. At the end of the study total serum cholesterol and triglycerides decreased significantly by 10 and 30%, respectively (p less than 0.01). HDL increased in its cholesterol content by 33% reaching a value of 45.4 +/- 9.8 mg/dl (mean +/- SD, p less than 0.01) as well as in its apolipoprotein A1 (Apo A1) content (13%, p less than 0.02). HDL2 subfraction also rose in cholesterol and in Apo A1: by 90 and 38%, respectively (p less than 0.01). HDL3 subfraction rised only its cholesterol content by 24% (p less than 0.01). Tch/HDLch ratio was significantly reduced (p less than 0.01) to a value of 4.6 +/- 0.9 (mean +/- SD). Total serum Apo B diminished by 14% (p less than 0.01). No adverse effects were observed during the follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)